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LEARNING OBJECTIVES

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After completing this case study, the reader should be able to:

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  • Identify signs and symptoms of common problems in patients with cystic fibrosis (CF).

  • Develop an antimicrobial therapy plan and appropriate monitoring strategy for treatment of an acute pulmonary exacerbation in CF.

  • Devise treatment strategies for common complications of drug therapy in patients with CF.

  • Provide education on aerosolized medications to patients with CF, including appropriate instructions for dornase alfa and inhaled tobramycin.

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PATIENT PRESENTATION

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Chief Complaint

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As reported by patient’s father: “My daughter’s experiencing shortness of breath, fast breathing, increasing cough and sputum production, and decreased energy, and she has a poor appetite.”

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HPI

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Jenna O’Mally is a 7-year-old girl with a lifetime history of CF; she was diagnosed with CF at birth after presenting with meconium ileus. She had been doing well until 4 weeks ago, when she developed cold-like symptoms, with a runny nose, dry cough, sore throat, and subjective fever. She was seen at her local pediatrician’s office and prescribed a 5-day course of azithromycin suspension 200 mg/5 mL, 160 mg (10 mg/kg) PO on Day 1 and 80 mg (5 mg/kg) PO daily on Days 2–5 for possible pneumonia. After completing the antibiotic course, Jenna was not feeling any better. Father called the pulmonary clinic regarding her symptoms, and Jenna’s pulmonologist called in a prescription to a local pharmacy for ciprofloxacin suspension 250 mg/5 mL, 325 mg PO BID (~40 mg/kg per day), and prednisolone syrup 15 mg/5 mL, one teaspoonful PO twice daily. Father was also instructed to perform three chest physiotherapy sessions (vest treatments) per day and increase her hypertonic saline schedule from once per day to twice daily with her vest treatments. The patient now presents to the pulmonary clinic for a follow-up to her outpatient treatment course. She describes worsening shortness of breath and chest pain, lung and sinus congestion, poor appetite, and severe fatigue. Father reports increasing cough productive of very dark green sputum but no fever. The patient has lost 2 lb since her last clinic visit and has missed 7 days of school. Her oxygen saturation is 88% in clinic on room air, and she was immediately placed on 1 L of O2 by nasal cannula.

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PMH

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  • CF (Phe508del/G551D)

  • Significant for seven hospitalizations for acute pulmonary exacerbations of CF and two hospitalizations for distal intestinal obstruction syndrome (DIOS) since her initial NICU stay at birth; last hospitalization was 4 months ago

  • Sinus surgery × 2, last 1 year ago

  • Pancreatic insufficiency

  • Poor nutritional status

  • Recurrent constipation/DIOS

  • Pulmonary changes c/w long-standing CF with mild bronchiectasis

  • Seasonal allergies

  • Asthma

  • Broken clavicle previous summer after falling from a tree

  • ADHD

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FH

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Both parents are alive and generally well (father has hypercholesterolemia). Jenna has an older half-brother ...

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